Takotsubo’s syndrome is a rare acute reversible heart failure, where the pathophysiology is not fully understood. It is being increasingly diagnosed in varied clinical contexts, which can result in atypical presentations in the context of surgical or anaesthetic stress. We discuss the case of a 22-year-old woman who developed cardiogenic shock and impaired left ventricular function after an elective gynaecological procedure. She had a rapid recovery and a follow-up cardiac MRI confirmed Takotsubo’s syndrome.
- cardiovascular medicine
- heart failure
- emergency medicine
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Takotsubo’s syndrome is a rare acute reversible heart failure, where the pathophysiology is not fully understood. It is being increasingly diagnosed in varied clinical contexts, which can result in atypical presentations in the context of surgical or anaesthetic stress. Patients presenting with heart failure and cardiogenic shock, especially in the perioperative period present a diagnostic challenge. When managing patients with cardiogenic shock multidisciplinary team involvement with clear communication between specialties is essential. The use of bedside testing and early imaging facilitated a diagnosis that might otherwise have been overlooked.
We report the case of a 22-year-old woman who develops cardiogenic shock and impaired left ventricular (LV) function after an elective gynaecological procedure. She has a rapid recovery and a follow-up cardiac (MRI) confirmed Takotsubo’s syndrome.
A 22-year-old woman attended the day surgery unit for an elective evacuation of retained products of conception following an uneventful surgical termination of pregnancy under general anaesthesia, 2 weeks prior. She had no significant previous medical history, no allergies, no regular medications and was a non-smoker with no recreational drug use.
She was induced with propofol and alfentanyl. A supraglottic airway (iGel) was placed and anaesthesia maintained with propofol boluses. On transfer to theatre she developed a transient rash, but intraoperative observations were stable and she was easy to ventilate. Additional medications included ondansetron 8 mg, cyclizine 50 mg, tranexamic acid 1 g, paracetamol 1 g, paracoxib 40 mg and doxapram.
She was extubated and transferred to recovery. Two hours later she was reviewed as her oxygen saturations (SpO2) were 90%–95% on 2 L of oxygen and her heart rate was 100 beats/min (bpm). She was otherwise awake and symptom-free, with no chest pain, respiratory distress or bleeding. She was deemed not suitable for discharge and planned for admission and review overnight.
An hour later a periarrest call was issued from recovery as the patient was unresponsive with unrecordable SpO2 and systolic blood pressure (BP) of 70 mm Hg. This was attended by anaesthetics, intensive care and emergency department (ED) teams. Oxygenation improved with airway manoeuvres and she was transferred to ED resuscitation.
On re-examination she had a patent airway but was in respiratory distress. Her respiratory rate was 26 with SpO2 of 89% on 15 L of oxygen via non-rebreather mask, falling to <80% of oxygen. Her chest was clear and both heart sounds were present with no added sounds. She was tachycardic (110 bpm) and hypotensive (90/50 mm Hg). She never reported any chest pain. Her Glasgow Coma Scale (GCS) was 14, with no focal neurology and equal, reactive pupils.
Her serial 12-lead electrocardiograms (ECG) were normal with no ST segment changes. An arterial blood gas showed type 1 respiratory failure with a lactate of 2.5 mg/dL. Her haemoglobin was 117 g/L, platelet count 240×109/L and renal function normal. Her initial high-sensitivity troponin was significantly raised (1321 g/L; normal <16 g/L) and brain natriuretic peptide (BNP) 47 pg/mL.
Chest X-ray and subsequent contrast CT chest, to exclude pulmonary embolus, showed widespread pulmonary oedema. She was started on non-invasive ventilation (NIV) and intravenous furosemide; her BP and oxygenation subsequently improved. A bedside echocardiogram (ECHO) performed by the cardiology registrar showed severely impaired LV function, in-keeping with dilated cardiomyopathy.
Her age and lack of previous medical history combined with the rapid deterioration meant the differential diagnoses explored were broad. The tachycardia and tachypnoea made pneumothorax or pulmonary embolism likely possibilities. Negative pressure pulmonary oedema was considered but thought unlikely as the iGel contained a bite block. The pulmonary oedema seen on CT led to consideration of less common cardiac causes that might present acutely without ECG changes, such as myocarditis. It was not until the ECHO demonstrated severely impaired LV function was Takotsubo’s syndrome considered.
Outcome and follow-up
She was admitted to the intensive care unit for respiratory support, cardiac monitoring and diuresis. Overnight her condition improved; NIV was weaned to high flow oxygen and she was stable on room air by morning. A repeat ECHO on day 2 showed an improved LV ejection fraction of 56%. She was transferred to a cardiac centre for cardiac MRI which confirmed Takotsubo’s syndrome, predominantly affecting the mid LV. She was commenced on regular bisoprolol and lisinopril, and discharged home on day 3. She remained well under regular cardiology review and is predicted to make a full recovery.
Takotsubo’s syndrome, also called stress-induced cardiomyopathy or broken heart syndrome, is an acute reversible heart failure, commonly occurring after physical or emotional stress. It was first diagnosed in 1990 and named after Japanese octopus pots, due to the appearance of the hypokinetic left ventricle on ECHO. Takotsubo’s syndrome is being increasingly diagnosed in hospitalised patients, partly due to improved access to coronary angiography helping to differentiate this condition from acute coronary syndrome (ACS). The increasing ‘atypical’ presentations is challenging the assumption that Takotsubo’s syndrome is a disease of postmenopausal women.
Primary versus secondary Takotsubo’s syndrome
Primary Takotsubo’s syndrome is when acute cardiac symptoms, such as chest pain, breathlessness or palpitations, cause the patient to seek medical care. Emotional or physical stress precedes the onset in 45%–85% of cases.1 Secondary Takotsubo’s syndrome is the development of the condition in already hospitalised patients, where the sudden activation of the sympathetic system precipitates Takotsubo’s syndrome.2 It tends to present with heart failure (66%), ACS (36%), cardiogenic shock and arrest. These patients tend to be younger than those with primary Takotsubo’s syndrome.2
The general population incidence is estimated at 2–9 in 100 000.3 Studies suggest that 1%–2% of patients presenting with ACS have Takotsubo’s syndrome, and this is likely an under diagnosis.4 Postmenopausal women represent 90% of cases, with <10% of patients aged <50 years old.
Several case series report that 3%–23% of cases of Takotsubo’s appear to have been triggered by surgical procedures; however, it is unclear if the trigger is the surgical condition, operation or anaesthesia.5 6 Between 2000 and 2016, Hessel et al identified 131 published cases in the perioperative period and estimated an incidence of 1 in 6700 surgeries. Of these cases 58% occurred postoperatively, 37% during anaesthesia and 5% at induction. No precipitating factor was identified in 44% of these cases, compared with the international registry (28.5%).1 5
Takotsubo’s syndrome is a complex systemic and psychological response to acute stress resulting in myocardial stunning. It is often classified as a cardiomyopathy despite there being no primary disease of the cardiac muscle. The exact pathophysiology is unknown, but the most commonly accepted hypothesis is that it is the result of excessive sympathetic stimulation and catecholamine release. This causes direct myocardial damage due to the intracellular effects of excess catecholamines and ischaemic effects secondary to microvascular dysfunction.7 8 The regional distribution of adrenergic receptors may explain the distribution of regional wall motion abnormality (RWMA). Iatrogenic Takotsubo’s syndrome has been described after the administration of sympathomimetic drugs such as local anaesthetics with epinephrine or during dobutamine stress ECHO.8 Another theory is that transient ischaemia, due to microvascular dysfunction, coronary spasm or myocardial infarction with spontaneous recanalisation may play a role in myocardial injury. There is likely a genetic component, for example, differences in the alpha and beta adrenoreceptors, explaining the susceptibility of some individuals.1
The most widely used diagnostic standard is the 2008 Mayo clinic criteria (box 1).9 Diagnosis requires an absence of coronary artery disease, with the RWMA usually extending beyond a single vascular territory. Takotsubo’s commonly mimics ACS and coronary angiography is the gold standard to exclude coronary artery disease; however, cardiac MRI or CT can aid diagnosis.2
Mayo clinic diagnostic criteria for Takotsubo’s syndrome9
1. Transient left ventricular systolic dysfunction (hypokinesis, akinesis, or dyskinesis). The wall motion abnormalities are typically regional and extend beyond a single coronary vessel distribution. A stressful trigger is often, but not always present.
2. Absence of obstructive coronary disease or angiographic evidence of acute plaque rupture.
3. New electrocardiographic abnormalities (either ST-segment elevation and/or T wave inversion) or modest elevation in cardiac troponin.
4. Absence of pheochromocytoma or myocarditis
ECG changes are seen in 95% of patients in first 12 hours, commonly: ST elevation, ST depression, left bundle branch block, deep T-wave inversion, QTc prolongation and tachyarrhythmias. A small minority, as in this case, presented with normal ECG.7
During the acute phase there is a small troponin rise and significant naturietic peptide increase. The troponin rise is proportionally smaller than that typically seen during myocardial infarction.2
Early transthoracic ECHO is essential in assessing LV function and potential complications. RWMA may recover within hours and are easily missed if imaging is delayed. Where available cardiac MRI should be performed within 7 days, as it provides superior imaging to ECHO and the patterns of oedema on T2 weighted images can help distinguish Takotsubo’s syndrome from myocardial infarction.7
Many patients make a spontaneous recover but 50% experience complications.2 Heart failure is most common, occurring in 12%–45% of cases.3 5 10 Other complications include right ventricular involvement, LV outflow tract obstruction, mitral regurgitation, cardiogenic shock, arrhythmias and thrombus formation. The 5-year recurrence rate is 5%–22%, and 5-year mortality 3%–17%.1
The complications rate, morbidity and mortality are higher in patients with secondary Takotsubo’s syndrome. The overall hospital mortality is 1%–4.5% and this is increased 2-fold to 10-fold for patients with secondary Takotsubo’s syndrome.10–12 Brinjiki et al noted the mortality was twice as high in cases associated with operating room procedures.3 Secondary Takotsubo’s syndrome is associated with a lower ejection fraction, thought to be a risk factor for adverse events and increased mortality.13
The goal of care is supportive and complication prevention. There are no randomised controlled trials to support different treatment options; consequently management strategies are based on level C evidence. Diagnostic algorithms have been produced by the European Society of Cardiology to guide treatment and stratify risk.2
In mild cases medical management, including beta blockers, may be sufficient. Higher risk patients should be monitored in level 2 environment as life-threatening complications peak 72 hours after presentation.2
In cardiogenic shock, vasopressors and inotropes are considered contraindicated as they may worsen cardiac dysfunction. Evidence from the international Takotsubo’s registry indicates that sympathomimetics are strong independent risk factors for mortality. Deteriorating patients with multiorgan failure require early mechanical support such as left ventricular assist device (LVAD) or extracorporeal membrane oxygenation (ECMO). If unavailable, levosimendan can be used as a catecholamine sparing agent.
Recovery depends on disease severity. Symptomatic recovery begins within the first few hours and is complete within a week. LV ejection fraction usually recovers within 12 weeks and ECG changes can take up to 12 months to resolve. All patients should be followed up within 6 months with repeat cardiac imaging to confirm resolution of LV dysfunction.2
In patients with a history of Takotsubo’s syndrome presenting for surgery it is important to avoid physiological stress in the perioperative period. Causes of perioperative Takotsubo’s syndrome identified in the literature are pain, anxiety, respiratory distress secondary to residual neuromuscular block and epinephrine administration. Anxiolysis, adequate analgesia, avoidance of catecholamines and regional anaesthesia have all been suggested; however, the best anaesthetic technique has not been established.
Takotsubo’s is incompletely understood, with a lack of randomised controlled trials to guide management. The diagnosis of Takotsubo’s syndrome is increasing and in wider clinical contexts, including the perioperative period, which can be diagnostically challenging. Clinicians should be aware of the different presenting symptoms of primary and secondary Takotsubo’s syndrome, and include secondary Takotsubo syndrome in their differential for perioperative patients presenting with heart failure and cardiogenic shock.
Takotsubo’s syndrome is an acute and reversible heart failure, occurring after physical or emotional stress.
Takotsubo’s syndrome is increasingly being diagnosed in a variety of clinical contexts.
The exact aetiology of Takotsubo’s syndrome is unclear, and not all patients have a clearly defined trigger, but catecholamines may play a central role.
Presenting symptoms differ between primary and secondary Takotsubo’s syndrome.
Clinicians should include secondary Takotsubo’s syndrome in the differential diagnosis for perioperative patients presenting with heart failure and cardiogenic shock.
Availability of cardiac imaging, including bedside ECHO, coronary angiography and cardiac MRI, is increasing the diagnosis of Takotsubo’s syndrome
Contributors TD took the lead in writing the manuscript. TD and RS provided critical feedback and helped in editing the manuscript. SP supervised the project.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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